Early Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis: A Narrative Review

Pulm Ther. 2023 Jun;9(2):177-193. doi: 10.1007/s41030-023-00216-0. Epub 2023 Feb 11.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease of unknown aetiology. Patients typically present with symptoms of chronic dyspnoea and cough over a period of months to years. IPF has a poor prognosis, with an average life expectancy of 3-5 years from diagnosis if left untreated. Two anti-fibrotic medications (nintedanib and pirfenidone) have been approved for the treatment of IPF. These drugs slow disease progression by reducing decline in lung function. Early diagnosis is crucial to ensure timely treatment selection and improve outcomes. High-resolution computed tomography (HRCT) plays a major role in the diagnosis of IPF. In this narrative review, we discuss the importance of early diagnosis, awareness among primary care physicians, lung cancer screening programmes and early IPF detection, and barriers to accessing anti-fibrotic medications.

Keywords: Anti-fibrotic medications; Early diagnosis; Idiopathic pulmonary fibrosis.

Publication types

  • Review